Jove
Visualize
Contact Us
JoVE
x logofacebook logolinkedin logoyoutube logo
ABOUT JoVE
OverviewLeadershipBlogJoVE Help Center
AUTHORS
Publishing ProcessEditorial BoardScope & PoliciesPeer ReviewFAQSubmit
LIBRARIANS
TestimonialsSubscriptionsAccessResourcesLibrary Advisory BoardFAQ
RESEARCH
JoVE JournalMethods CollectionsJoVE Encyclopedia of ExperimentsArchive
EDUCATION
JoVE CoreJoVE BusinessJoVE Science EducationJoVE Lab ManualFaculty Resource CenterFaculty Site
Terms & Conditions of Use
Privacy Policy
Policies

Related Concept Videos

Animal Mitochondrial Genetics02:59

Animal Mitochondrial Genetics

9.3K
Among all the organelles in an animal cell, only mitochondria have their own independent genomes. Animal mitochondrial DNA is a double-stranded, closed-circular molecule with around 20,000 base pairs. Mitochondrial DNA is unique in that one of its two strands, the heavy, or H, -strand is guanine rich, whereas the complementary strand is cytosine rich and called the light, or L, -strand. Compared to nuclear DNA, mitochondrial DNA has a very low percentage of non-coding regions and is marked by...
9.3K
Comparing Mitochondrial, Chloroplast, and Prokaryotic Genomes02:16

Comparing Mitochondrial, Chloroplast, and Prokaryotic Genomes

16.2K
The present-day mitochondrial and chloroplast genomes have retained some of the characteristics of their ancestral prokaryotes and also have acquired new attributes during their evolution within eukaryotic cells. Like prokaryotic genomes, mitochondrial and chloroplast genomes neither bind with histone-like proteins nor show complex packaging into chromosome-like structures, as observed in eukaryotes. Unlike mitotic cell divisions observed in eukaryotic cells, mitochondria and chloroplasts...
16.2K
Export of Mitochondrial and Chloroplast Genes02:19

Export of Mitochondrial and Chloroplast Genes

4.2K
A eukaryotic cell can have up to three different types of genetic systems: nuclear, mitochondrial, and chloroplast. During evolution, organelles have exported many genes to the nucleus; this transfer is still ongoing in some plant species. Approximately 18% of the Arabidopsis thaliana nuclear genome is thought to be derived from the chloroplast’s cyanobacterial ancestor, and around 75% of the yeast genome derived from the mitochondria’s bacterial ancestor. This export has occurred...
4.2K
Introduction to Fibroblasts01:09

Introduction to Fibroblasts

4.0K
Rudolph Virchow discovered spindle-shaped cells called fibroblasts in 1858. Inactive fibroblasts, called fibrocytes, become activated by various stimuli, such as growth factors and inflammatory cytokines. Activated fibroblasts play a crucial role in wound healing, inflammation, formation of new blood vessels, and cancer progression. Uncontrolled activation of fibroblasts results in fibrosis, the excess deposition of fibrous tissue, which can lead to scarring and affect normal organs. This...
4.0K
Multiple Allele Traits01:49

Multiple Allele Traits

38.1K
The Concept of Multiple Allelism
38.1K
The Inner Mitochondrial Membrane01:28

The Inner Mitochondrial Membrane

4.7K
The inner mitochondrial membrane is the primary site of ATP synthesis. The inner membrane domain that forms a smooth layer adjacent to the outer membrane is called the inner boundary membrane. This domain contains membrane transporters that drive metabolites in and out of the mitochondria.  In contrast, the inner membrane network that invaginates into the matrix space is called the cristae membrane. This domain accounts for principle mitochondrial function as it accommodates the protein...
4.7K

You might also read

Related Articles

Articles linked to this work by shared authors, journal, and citation graph.

Sort by
Same author

Metabolic brain imaging in genetic Parkinson's disease: from genes to network trajectories.

Parkinsonism & related disorders·2026
Same author

Movement disorders in GLUT1 deficiency syndrome: a systematic review of the literature.

Journal of neurology·2026
Same author

Essential genetic testing in movement disorders - results from a Delphi study.

Parkinsonism & related disorders·2026
Same author

Age-Specific Parkinson Disease Risk in Gaucher Disease Type 1: Data From the ICGG Gaucher Registry.

Neurology·2026
Same author

Rethinking Mitochondrial Parkinson's Disease in the α-Synuclein Seed Amplification Assays Era.

Movement disorders : official journal of the Movement Disorder Society·2026
Same author

Sex Differences in Levodopa-Sparing Effect of Safinamide: Post-hoc Findings from a Multicenter, Longitudinal, Case-Control Study.

Movement disorders clinical practice·2026

Related Experiment Video

Updated: Feb 4, 2026

An In Vitro Approach to Study Mitochondrial Dysfunction: A Cybrid Model
06:05

An In Vitro Approach to Study Mitochondrial Dysfunction: A Cybrid Model

Published on: March 9, 2022

4.4K

Mitochondrial dysfunction in fibroblasts of Multiple System Atrophy.

Giacomo Monzio Compagnoni1, Giulio Kleiner2, Andreina Bordoni1

  • 1IRCCS Foundation Ca' Granda Ospedale Maggiore Policlinico, Dino Ferrari Center, Neuroscience Section, Department of Pathophysiology and Transplantation, University of Milan, Milan, Italy.

Biochimica Et Biophysica Acta. Molecular Basis of Disease
|September 27, 2018
PubMed
Summary

Multiple System Atrophy (MSA) involves mitochondrial dysfunction, including impaired respiratory chain activity and mitophagy. These findings in cellular models offer insights into MSA pathogenesis and potential therapeutic targets for this neurodegenerative disease.

Keywords:
Cellular modelsFibroblastsMitochondriaMultiple System Atrophy

More Related Videos

The Use of Primary Human Fibroblasts for Monitoring Mitochondrial Phenotypes in the Field of Parkinson's Disease
15:09

The Use of Primary Human Fibroblasts for Monitoring Mitochondrial Phenotypes in the Field of Parkinson's Disease

Published on: October 3, 2012

17.4K
Assessment of Mitochondrial Health in Cancer-Associated Fibroblasts Isolated from 3D Multicellular Lung Tumor Spheroids
10:26

Assessment of Mitochondrial Health in Cancer-Associated Fibroblasts Isolated from 3D Multicellular Lung Tumor Spheroids

Published on: October 21, 2022

2.5K

Related Experiment Videos

Last Updated: Feb 4, 2026

An In Vitro Approach to Study Mitochondrial Dysfunction: A Cybrid Model
06:05

An In Vitro Approach to Study Mitochondrial Dysfunction: A Cybrid Model

Published on: March 9, 2022

4.4K
The Use of Primary Human Fibroblasts for Monitoring Mitochondrial Phenotypes in the Field of Parkinson's Disease
15:09

The Use of Primary Human Fibroblasts for Monitoring Mitochondrial Phenotypes in the Field of Parkinson's Disease

Published on: October 3, 2012

17.4K
Assessment of Mitochondrial Health in Cancer-Associated Fibroblasts Isolated from 3D Multicellular Lung Tumor Spheroids
10:26

Assessment of Mitochondrial Health in Cancer-Associated Fibroblasts Isolated from 3D Multicellular Lung Tumor Spheroids

Published on: October 21, 2022

2.5K

Area of Science:

  • Neuroscience
  • Cell Biology
  • Biochemistry

Background:

  • Multiple System Atrophy (MSA) is a severe neurodegenerative disorder with unknown pathogenic mechanisms.
  • Understanding the molecular basis of MSA is crucial for developing effective treatments.

Purpose of the Study:

  • To investigate the causative mechanisms of MSA, focusing on mitochondrial dysfunction.
  • To establish a cellular model of MSA using primary fibroblast cultures.

Main Methods:

  • Analyzed fibroblasts from MSA patients (MSA-P, MSA-C) and healthy controls.
  • Assessed mitochondrial functioning, including respiratory chain activity, mitophagy, and autophagic activity.
  • Quantified Coenzyme Q10 levels and expression of its biosynthesis enzymes.

Main Results:

  • Observed impaired respiratory chain activity (Complex II) and reduced Coenzyme Q10 levels in MSA fibroblasts.
  • Demonstrated impaired mitophagy and reduced basal autophagic activity (LC3 II).
  • Found increased mitochondrial mass in MSA-C patients and alterations in mitochondrial DNA content.

Conclusions:

  • Mitochondrial dysfunction, including impaired respiratory chain, mitophagy, and Coenzyme Q10 biosynthesis, plays a significant role in MSA pathogenesis.
  • The study provides insights into MSA mechanisms and identifies potential therapeutic targets for this incurable disorder.